Abstract：
:Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (≥ 20%) and absolute number (≥ 2 × 10(9)/l) of plasma cells in the peripheral blood. It is proposed that the thresholds for diagnosis be re-examined and consensus recommendations are made for diagnosis, as well as, response and progression criteria. Induction therapy needs to begin promptly and have high clinical activity leading to rapid disease control in an effort to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem cell transplantation if feasible. Allogeneic transplantation can be considered in younger patients. Prospective multicenter studies are required to provide revised definitions and better understanding of the pathogenesis of PCL.
journal_name
Leukemiajournal_title
Leukemiaauthors
Fernández de Larrea C,Kyle RA,Durie BG,Ludwig H,Usmani S,Vesole DH,Hajek R,San Miguel JF,Sezer O,Sonneveld P,Kumar SK,Mahindra A,Comenzo R,Palumbo A,Mazumber A,Anderson KC,Richardson PG,Badros AZ,Caers J,Cavo M,Ledoi
10.1038/leu.2012.336subject
Has Abstractpub_date
2013-04-01 00:00:00pages
780-91issue
4eissn
0887-6924issn
1476-5551pii
leu2012336journal_volume
27pub_type
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